WASHINGTON – Endogenous Cushing’s syndrome, a rare hormonal disorder, is associated with a threefold increase in mortality, primarily from cardiovascular disease and infection, according to a study whose results will be presented at ENDO 2021, annual meeting Endocrine Society.
The research, according to the study’s authors, is by far the largest systematic and meta-analysis of studies of endogenous (meaning “inside your body”) Cushing syndrome. Although Cushing’s syndrome is usually caused by external factors – taking cortisol-like drugs such as prednisone – the endogenous type occurs when the body makes too much of the hormone cortisol, which affecting several bodily systems.
The lack of accurate data on mortality and specific causes of death in people with endogenous Cushing syndrome, said the study’s lead author, Padiporn Limumpornpetch, MD, an endocrinologist from Prince Songkla University, Thailand and Ph.D. student at the University of Leeds in Leeds, UK The study analyzed death data from more than 19,000 patients in 92 studies published through January 2021.
“Our findings found that mortality rates have fallen since 2000 but are still inappropriately high,” Limumpornpetch said.
Cushing’s syndrome affects many parts of the body as cortisol counteracts stress, maintains blood pressure and cardiovascular function, regulates blood sugar and monitors the immune system. The most common cause of endogenous Cushing syndrome is a tumor of the pituitary gland called Cushing’s disease, but another cause of abnormal tumor is usually of the adrenal glands called adrenal Cushing syndrome. All patients in this study had noncancerous tumors, according to Limumpornpetch.
Overall, the proportion of deaths from each study coach was 5 percent, the researchers said. The normal mortality ratio – the ratio of deaths observed in the study group to expected deaths in the general population by age and sex – was 3: 1, indicating a threefold increase in their deaths, she said.
This mortality ratio was reported to be higher in patients with Cushing’s adrenal syndrome versus Cushing’s disease and in patients with active versus Cushing’s disease. The normal mortality ratio was also worse in patients with Cushing’s disease with larger tumors versus very small tumors (macroadenomas versus microadenomas).
On the positive side, mortality rates were lower after 2000 compared to then, which Limumpornpetch attributed to advances in diagnosis, procedures, and surgical care.
More than half of the deaths studied were due to heart disease (24.7 per cent), diseases (14.4 per cent), cerebrovascular diseases such as stroke or aneurysm (9.4 per cent) or blood clots in vein, called thromboembolism (4.2 percent).
“Causes of death highlight the need for aggressive management of cardiovascular risk, prevention of thromboembolism and good infection control and underscore the need to achieve disease relief, normalizing cortisol levels,” she said.
Surgery is the primary treatment for Cushing’s syndrome. If surgery to remove the tumor does not remove the infection, there are other treatments available, such as medications.
Study co-author Victoria Nyaga, Ph.D., of the Belgian Cancer Center in Brussels, Belgium, developed the Metapreg statistical analysis program used in this study.
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